1. Cerebral Palsy Cerebral palsy (CP) is a diagnostic term used to describe a group of motor syndromes resulting from disorders of early brain development. CP is caused by a broad group of developmental, genetic, metabolic, ischemic, infectious.

2. EPIDEMIOLOGY AND ETIOLOGY CP is the most common and costly form of chronic motor disability that begins in childhood with a prevalence of 2/1000. In 80% of cases, features were identified pointing to antenatal factors causing abnormal brain development

3. A substantial number of children with CP had congenital anomalies external to the central nervous system (CNS). Fewer than 10% of children with CP had evidence of intrapartum asphyxia. Intrauterine exposure to maternal infection (chorioamnionitis, inflammation of placental membranes, umbilical cord inflammation, foul- smelling amniotic fluid, maternal sepsis, temperature >38°C during labor, urinary tract infection) is associated with a significant increase in the risk of CP in normal birthweight infants.

4. About 10% are postnatal in origin. Preterm infants are especially vulnerable to brain damage from periventricular leucomalacia (PVL) secondary to ischaemia and/or severe intraventricular haemorrhage. The rise in survival of extremely preterm infants has been accompanied by an increase in survivors with cerebral palsy, although the number of such children is relatively small. Other postnatal causes are meningitis/encephalitis/encephalopathy, head trauma from accidental or non-accidental injury, symptomatic hypoglycaemia, hydrocephalus and hyperbilirubinaemia.

5. CLINICAL MANIFESTATIONS. Many children who develop cerebral palsy will have been identified as being at risk in the neonatal period. Early features of cerebral palsy are: abnormal limb tone and limb and/or trunk posture in infancy with delayed motor milestones may be accompanied by slowing of head growth feeding difficulties, with oromotor incoordination, slow feeding, gagging and vomiting abnormal gait once walking is achieved asymmetric hand function before 12 months of age.

12. Types of Cerebral Palsy

21. TREATMENT A team of physicians from various specialties, as well as occupational and physical therapists, speech pathologists, social workers, educators, and developmental psychologists provide important contributions to the treatment of these children. Parents should be taught how to work with their child in daily activities such as feeding, carrying, dressing, bathing, and playing in ways that limit the effects of abnormal muscle tone. They also need to be instructed in the supervision of a series of exercises designed to prevent the development of contractures, especially a tight Achilles tendon. There is no proof that physical or occupational therapy prevents development of CP in infants at risk or that it corrects the neurologic deficit, but evidence shows that therapy optimizes the functioning of children to achieve their potential.

22. Medication Several drugs have been used to treat spasticity, including oral dantrolene sodium, the benzodiazepines, and baclofen. These medications have modest beneficial effects in some patients, but can also cause side effects such as sedation for benzodiazepines and lowered seizure threshold for baclofen. Intrathecal baclofen has been used successfully in selected children with severe spasticity. This therapy requires a team approach and constant follow-up for complications of the infusion pumping mechanism and infection. Botulinum toxin injected into specific muscle groups for the management of spasticity shows a very positive response in many patients. Botulism toxin injected into salivary glands may also help reduce the severity of drooling, which is seen in 10 – 30% of patients with CP and has been traditionally treated with anticholinergic agents. Patients with rigidity, dystonia, and spastic quadriparesis sometimes respond to levodopa, and children with dystonia may benefit from carbamazepine or trihexyphenidyl.

23. HEREDITARY AND METABOLIC DEGENERATIVE DISEASES Degenerative diseases may affect gray matter (neuronal degenerative disorders), white matter (leukodystrophies), or specific, focal regions of the brain. Many white and gray matter degenerative illnesses result from enzymatic disorders within subcellular organelles, including lysosomes, mitochondria, and peroxisomes.

24. Gray matter degeneration (neuronal degeneration) is characterized early by dementia and seizures. This group of gray matter disorders, which cause slowly progressive loss of neuronal function, is separated into disorders with and disorders without accompanying visceromegaly (hepatosplenomegaly). Most are autosomal recessive traits except for Hunter syndrome (sex-linked recessive), Rett syndrome (sex-linked dominant), and the mitochondrial encephalopathies (nuclear or mitochondrial DNA defects).

25. Degenerative Diseases of the White Matter (Leukodystrophies) The prominent signs of diseases affecting primarily white matter are spasticity, ataxia, optic atrophy, and peripheral neuropathy. Seizures and dementia are late manifestations. Life expectancy ranges from months to a few years.

26. Mental retardation

27. Mental Retardation (Intellectual Disability) Mental retardation refers to a group of disorders that have in common deficits of adaptive and intellectual function and an age of onset before maturity is reached.

28. DEFINITION defines mental retardation as “ significantly sub-average general intellectual functioning, existing concurrently with deficits in adaptive behavior and manifested during the developmental period, that adversely affects a child's educational performance

29. Diagnostic Criteria for Mental Retardation A- Significantly sub-average intellectual functioning:an IQ score of ≈70 or below on an individually administered IQ test (for infants, a clinical judgment of significantly sub- average intellectual functioning). B- Concurrent deficits or impairments in present adaptive functioning (i.e., the meeting the standards expected for his or her age by his or her cultural group) in at least two of the following areas: communication, self-care, home living, social/interpersonal skills, use of community resources, self-direction, functional academic skills, work, leisure, health, and safety. C. The onset is before age 18 years.

30. CLINICAL MANIFESTATIONS Early diagnosis of mental retardation facilitates earlier intervention, realistic goal setting, easing of parental anxiety, and greater acceptance of the child in the community. Most children with intellectual disability 1st come to the pediatrician's attention in infancy because of dysmorphisms, associated dysfunctions, or failure to meet age-appropriate developmental milestones..

31. There are no specific physical characteristics of intellectual disability, but dysmorphisms are the earliest signs that bring children to the attention of the pediatrician. They may comprise a genetic syndrome such as Down syndrome or be isolated, as in microcephaly. Associated dysfunctions are neurologic disorders (seizures, cerebral palsy, autism) that are seen more frequently in conjunction with mental retardation than in the general population

32. Identification of Cause in Children with Severe Mental Retardation

33. 2 Trisomies 21,18,13, Klinefelter syndrome Chromosomal disorder 2121 Fragile X, Prader-Willi syndromeGenetic syndrome 9Hydrocephalus meningomyelocele, lissencephaly Developmental brain abnormality 8PKU, Tay-SachsInborn errors of metabolism/neurodegenerati ve disorder 4HIV, toxoplasmosis, rubella, CMV, syphilis, herpes simplex Congenital infections 6Environment, syndromic, or genetic Familial retardation 4HIE, meningitis, IVH, PVL, fetal alcohol syndrome Perinatal causes 5Trauma, meningitis, hypothyroidism Postnatal causes 2121 Cerebral palsyUnknown Total